Jackson R V, Fleming G A, Sussman C R, Atkinson A B, Decherney G S, Debold C R, Davis D R, Nicholson W E, Island D P, Rabin D
Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Aust Paediatr J. 1988;24 Suppl 1:70-3.
The response of plasma immunoreactive (IR)-ACTH, IR-beta-endorphin (beta-END) and IR-cortisol to insulin-induced hypoglycaemia, an acute stimulus to the pituitary corticotrophs through the central nervous system, and to synthetic ovine corticotrophin-releasing hormone (CRH), a direct corticotroph stimulator, were studied in normal males and males with myotonic dystrophy. Myotonics had an increased IR-ACTH and IR-beta-END response to hypoglycaemia and an increased IR-ACTH response to CRH compared with normals. Plasma IR-cortisol response were not different in either group of subjects to both stimuli. This neuroendocrine abnormality in myotonic dystrophy may represent a manifestation of the purported specific cell membrane defect underlying the disease. This is the first report of an abnormality in proopiomelanocortin peptide release in myotonic dystrophy.
在正常男性和患有强直性肌营养不良的男性中，研究了血浆免疫反应性（IR）-促肾上腺皮质激素（ACTH）、IR-β-内啡肽（β-END）和IR-皮质醇对胰岛素诱导的低血糖（一种通过中枢神经系统对垂体促肾上腺皮质激素细胞的急性刺激）以及对合成羊促肾上腺皮质激素释放激素（CRH，一种直接的促肾上腺皮质激素细胞刺激剂）的反应。与正常人相比，强直性肌营养不良患者对低血糖的IR-ACTH和IR-β-END反应增加，对CRH的IR-ACTH反应增加。两组受试者对两种刺激的血浆IR-皮质醇反应没有差异。强直性肌营养不良中的这种神经内分泌异常可能代表了该疾病潜在的特定细胞膜缺陷的一种表现。这是关于强直性肌营养不良中阿片促黑素皮质素原肽释放异常的首次报道。
